61例儿童支气管扩张症的病因及预后分析

The etiology and prognosis of 61 children with bronchiectasis

目的分析对儿童支气管扩张症的病因及预后情况。方法对2007年1月至2018年3月在本院住院治疗的61例支气管扩张症患儿的临床资料及预后进行回顾性分析。结果有36例支气管扩张症儿童的病因明确,其中感染后的支气管扩张症11例、原发性纤毛运动障碍综合征3例、肺囊性纤维化3例、X连锁无丙种球蛋白血症1例、低丙种球蛋白血症2例、高免疫球蛋白E综合征1例、结节病1例、先天性气管或支气管发育不良1例、其他因素引起的支气管扩张症13例,病因不明确者25例。80.3%(49例)的患儿支气管扩张表现为弥漫性支气管扩张;91.8%(56例)的患儿合并现症感染。随访成功的41例患儿中34例病情有改善,4例死亡。结论儿童支气管扩张症大部分是有因可寻的,其常见病因包括感染后、免疫缺陷及黏液清除功能异常等,经过积极、针对病因的治疗后支气管扩张症患儿生活质量及病情可有所改善。

Objective To increase the understanding of etiological and prognostic characteristics of bronchiectasis in children. Method 61 patients with bronchiectasis confirmed by computed tomography, who had been admitted to our hospital from January 2007 to March 2018, were retrospectively analyzed the patient＇s clinical features in hospital and prognosis after discharge. Result Of the 61 patients with bronchiectasis, 11 were after lung infections, 3 were primary ciliary dyskinesia and other 3 were cystic fibrosis, 5 were primary immunodeficiency diseases（including 1 case of X-linked globulin deficiency, 2 cases of hypogammaglobulinemia, 1 case of hyper Ig E syndrome and 1 case of sarcoidosis）, 1 was congenital hypoplastic trachea or bronchus, 13 were for other diseases that caused bronchiectasis. There were also 25 cases with unknown etiology. 80.3% of the 61 cases were all appeared as diffused bronchiectasis and 91.8%（56 cases） of them occurred to have current infection. Among the 41 telephone follow-up patients, 34 cases had progressive improvements in the quality of life and clinical manifestations, and 4 cases were died for their underlying diseases. Conclusion Most bronchiectasis in children can be found the underlying etiologies, and pulmonary infection,immunodeficiency diseases as well as mucus clearance dysfunction were the three main etiology for bronchiectasis. The quality of life and the clinical condition of children with bronchiectasis were improved after active and targeted treatment.